幕上原始神经外胚叶肿瘤临床病理学初探

目的 探讨原发于颅内幕上的原始神经外胚叶肿瘤（PNETs）的临床病理特征、组织学发生及生物学行为。方法 采用组织病理学、免疫组化标记对9例原发于幕上的PNETs进行研究。结果 肿瘤组织主要由小圆形细胞组成．部分为梭形细胞．细胞密集成巢，由纤维结缔组织分隔成小叶状，核染色质颗粒状，核仁不明显，多数可见核分裂象，形成Homer Wright菊形团。免疫组化标记显示神经特异性烯醇化酶阳性9例，突触素阳性5例，部分瘤组织中波形蛋白、胶质纤维酸性蛋白散在阳性；神经微丝蛋白、白细胞共同抗原、细胞角蛋白呈阴性。结论PNETs属胚胎源性神经上皮肿瘤，具高度恶性，其诊断除了病理形态学观察外，免疫组化检查必不可少。

A Preliminary Clinicopathological Study of Supratentorial Primitive Neuroectodermal Tumors: a Report of 9 Cases

Objective To explore clinicopathological features,histogenesis and biological behavior of sup-ratentorial primitive neuroectodermal tumors(SPNET).Methods Nine cases of SPNET were studied by histopathological and immunohistochemical stainings.Results The biopsy showed fibrous bands surrounding nests of small round cell or spindle cell.Homer Wright rosettes could be found.There were granular nuclear chromatin and obscure nucleoli in the tumor cells.Immunohistochemical staining showed positive staining for neuron specific enolase in 9 cases,synaptophysin in 5 cases,glial fibrillary acidic protein in 4 cases and vi-nentmin in 1 case,and negative staining for neurofilament,leukocyte comman antigen and cell kerawtin in all the tumors.Conclusions SPNET is a rare and highly malignant tumor of soft tissue,and its diagnosis must be made by immunohistochemistry.